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The RGCs axons form the optic nerve. Therefore, the disease can be considered of the central nervous system. [ 2 ] Dominant optic atrophy was first described clinically by Batten in 1896 and named Kjer’s optic neuropathy in 1959 after Danish ophthalmologist Poul Kjer , who studied 19 families with the disease. [ 3 ]
The optic nerve can be damaged when exposed to direct or indirect injury. Direct optic nerve injuries are caused by trauma to the head or orbit that crosses normal tissue planes and disrupts the anatomy and function of the optic nerve; e.g., a bullet or forceps that physically injures the optic nerve.
The left optic nerve and the optic tracts. A Marcus Gunn pupil indicates an afferent defect, usually at the level of the retina or optic nerve. Moving a bright light from the unaffected eye to the affected eye would cause both eyes to dilate, because the ability to perceive the bright light is diminished. Specialty: Ophthalmology, Optometry
Chronic relapsing inflammatory optic neuropathy (CRION) is a form of recurrent optic neuritis that is steroid responsive and dependent. [1] Patients typically present with pain associated with visual loss. [1] CRION is a clinical diagnosis of exclusion, and other demyelinating, autoimmune, and systemic causes should be ruled out. [3]
The exact mechanism of optic nerve ischemia in these cases remains unclear, but contributing factors may include hypotension, anemia, hypoxia, and changes in the autoregulation of optic nerve arterial blood flow. The incidence of ischemic optic neuropathy leading to vision loss following general surgeries ranges between 0.1% and 0.002%.
This disease is a heterogenous group of inherited neuropathies, stemming from a MFN2 mutation, in which both motor and sensory nerves are affected, resulting in distal limb weakness, sensory loss, decreased deep tendon reflexes, and foot deformities. Affected individuals develop progressive optic nerve dysfunction starting later in childhood. [21]
Neuromyelitis optica spectrum disorders (NMOSD) are a spectrum of autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord . [1] [2] [3] Episodes of ON and myelitis can be simultaneous or successive. A relapsing disease course is common, especially in untreated patients. [1] [4]
Redundant fibroglial tissue also is seen in severe cases. Milder forms of dysplasia exhibit missing portions of the optic disc located in the optic nerve pit. The least severe form of papillorenal disease shown in the eye is the exiting of blood vessels from the periphery that do not disturb the shape of the eye.